Oxford Centre for Diabetes, Endocrinology and Metabolism, Churchill Hospital, University of Oxford, Oxford, OX3 7LE, UK.
Endocrine. 2012 Aug;42(1):69-73. doi: 10.1007/s12020-012-9646-7. Epub 2012 Mar 7.
Cushing's syndrome (CS) is a condition associated with high cortisol levels and affects around 0.7-2.4 cases per million population per year [1-3]. Approximately 80 % of cases of CS are secondary to excess adrenocorticotrophin (ACTH) secretion, while in around 20 % the primary abnormality lies in the adrenal, most often an adrenal adenoma or carcinoma. Of the ACTH-dependent causes, some 80-90 % are due to a pituitary adenoma-Cushing's disease-but in a significant proportion the cause is ectopic secretion from a non-pituitary source, the ectopic ACTH syndrome (EAS) [4]. The commonest source of ACTH secretion in these patients is a bronchial carcinoid. However, many other tumors are capable of secreting ACTH, and in most series a small but significant number are secondary to an ACTH-secreting pheochromocytoma. Amalgamating the data from 6 large series of patients with ectopic ACTH, pheochromocytoma was the source of ACTH secretion in 19 out of 363 patients (5.2 %) [5-11]. In this mini-review, we present a patient presenting to our department with a pheochromocytoma as well as Cushing's syndrome due to ectopic ACTH secretion by the pheochromocytoma, and we discuss published cases in the world literature to assess its significance. We emphasize the problems in the simultaneous management of these two serious endocrine conditions.
库欣综合征（CS）是一种与高皮质醇水平相关的疾病，每年每百万人中有 0.7-2.4 例[1-3]。大约 80%的 CS 病例继发于促肾上腺皮质激素（ACTH）分泌过多，而在大约 20%的病例中，原发性异常位于肾上腺，最常见的是肾上腺腺瘤或癌。在依赖 ACTH 的病因中，大约 80-90%是由于垂体腺瘤-库欣病引起的，但在很大一部分患者中，病因是来自非垂体来源的异位分泌，即异位 ACTH 综合征（EAS）[4]。在这些患者中，ACTH 分泌最常见的来源是支气管类癌。然而，许多其他肿瘤也能够分泌 ACTH，在大多数系列中，少数但有重要意义的患者是继发于 ACTH 分泌性嗜铬细胞瘤。综合 6 个大型异位 ACTH 患者系列的数据，嗜铬细胞瘤是 363 例患者中的 19 例（5.2%）ACTH 分泌的来源[5-11]。在这个小型综述中，我们介绍了一位因嗜铬细胞瘤引起的异位 ACTH 分泌而同时患有库欣综合征的患者，并讨论了世界文献中的已发表病例，以评估其意义。我们强调了同时管理这两种严重内分泌疾病的问题。
